Role of Echo in Connective Tissue Diseases

1
Role of Echo inConnective Tissue Diseases

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2
Connective Tissue Diseases

• Systemic lupus erythematosus
• Antiphospholipid antibody syndrome
• Ankylosing spondylitis
• Rheumatoid arthritis
• Scleroderma
• Polymyositis and dermatomyositis
• Mixed connective tissue disease

3
Cardiac Diseases in Pts with SLE

• Pericardial disease
• Pericarditis
• Valvular disease
• Fibrin deposits (Libman-Sacks)
• Fibrous thickening of leaflets and chordae
• Valvular regurgitation and/ or stenosis
• Infective endocarditis
• Coronary artery diseases
• Myocardial diseae
• Hypertrophy

4
Prevalence of Pericarditis in Pts with SLE

• Meta-analysis of 26 studies
• 
  (Mayo Clin Proc 199974255)
• Clinical or echo prevalence
• 595/2,147 (28)
• Autopsy prevelence
• 188/291 (65)
• Cardiac tamponade
• 16/2,147 (0.7)
• Echo prevalence
• 22 54 (Control 0 10)

5
Valvular Disease Associated with SLE

• Valve masses or
• Libman-Sacks vegetation
• Leaflet thickening
• Valvular regurgitation
• Valvular stenosis

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Libman-Sacks Vegetation
Cauliflower-like or flat, red multiple spreading
masses of 2 4 mm in diameter present on the
free margins or line of closure of the heart valve
Echo findings

• Prevalence
• TTE 10, TEE 30
• Mitral and aortic valves
• lt 1 cm2 in size
• Irregular borders
• Heterogenous echo density
• No independent motion
• Associated with thickening or regurgitation

(Cardiol Clin 199816531)
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Libman-Sacks Vegetation and MR
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Abnormal Leaflet Thickening in SLE

• Valve thickness gt 3 mm for MV and TV
• gt 2 mm for AV
• Prevalence
• TTE 30, TEE 50
• Mitral and aortic valves
• Generally diffuse but predominant on the mid and
  tip portions
• Commonly associated with valve regurgitation or
  valve masses or both
• Valve stenosis is rare (lt3).
• Leaflet calcification is uncommon.
• Involvement of the annular and subvalvular
  apparatus is rare (1).

9
Valvular Regurgitation in SLE

• The most frequent abnormality (up to 79)
• Moderate-to-severe regurgitation in 7 to 41
• MVgtTVgtAVgtPV
• Moderate or severe regurgitation is almost always
  accompanied by leaflet thickening.

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Differential Diagnosis Libman-Sacks vegetation

• Infective endocarditis (IE)
• Vibratory or rotatory motion independent of the
  leaflet motion
• Pseudoinfective endocarditis
• A clinical syndrome of active SLE mimics IE.
• Low WBC count
• Elevation of antiphospholipid Ab
• Negative or low positive CRP

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Differential Diagnosis Abnormal Leaflet
Thickening in SLE

• Rheumatic valvular disease
• Leaflet thickening localized to the leaflet tips
• Chordal thickening, fusion, tethered motion and
  calcification
• Age-related valvular disease
• Valve sclerosis is marked in the valve annulus.
• Frequently associated with calcification

12
Evolution of Valvular Disease in SLE

• Resolve (24), appear de novo (12), or persist
  but change over time (40)

Initial
2 mon later
20 mon later
(NEJM 19963351424)
13
Clinical Course of Pts with SLE and Valvular
DIsease

• Neither the presence nor the changes over time in
  valvular disease were temporarily associated with
  pts age or with the duration, activity,
  severity, or therapy of SLE
• 21 incidence of valve-related complications with
  a 5-yr F/U
• Symptomatic severe valvular regurgitation
• Infective endocarditis
• Ischemic stroke
• Vegetation, valvulitis, and LA thrombus in
  70 to 90
• Mortality
• 20 at 5 yrs
• Causes of death
• Refractory HF, infective endocarditis,
  complicated postoperative course and CVA

14
Therapy of Pts with SLE and Valvular Disease

• Prosthetic valve replacement or valve repair
• Higher morbidity and mortality of valve
  replacement
• Steroid or cytotoxic therapy has no effect on the
  presence or the evolution of SLE-associated
  valvular disease
• Antibiotic prophylaxis for dental or nonsterile
  procedures
• Antiplatelet therapy

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Antiphospholipid Antibody Syndrome

• Venous or arterial thrombosis, recurrent fetal
  loss, or thrombocytopenia accompanied by an
  increased levels of antiphospholipid Ab (aPLs)
• Primary or secondary (SLE)
• Valvular lesions
• Vegetation, thickening, or regurgitation
• Prevalence
• 32 to 38 in primary APS
• A significantly higher prevalence of valvular
  defects in SLE pts with aPLs
• Therapy
• Long-term, high intensity oral
  anticoagulation (INR ³3)

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Aortic Root Disease and Valve Disease Associated
with Ankylosing Spondylitis

• Pathology
• The inflammatory process predominantly of the
  adventitia and intima of the aortic root results
  in a fibroblastic reparative response and
  vascularized fibrous tissue thickening
• Aortic valvulitis
• Cusp thickening and retraction
• Thickening of the aorto-mitral junction or
  subaortic bump
• Proximal aortitis leading to aortic root
  thickening and dilation
• Aortic and mitral regurgitation

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Aortic Root Disease and Valve Disease Associated
with Ankylosing Spondylitis

• Prevalence
• Autopsy studies 24 to 100
• Echo studies
• TTE 8 to 31
• TEE 82 (control 27)
• Echo findings
• Nonspecific thickening of aortic and mitral
  valves
• Increased echogenicity of the posterior aortic
  wall and membraneous interventricular septum
• Mild-to-moderate aortic regurgitation

18
Aortic Root Disease and Valve Disease Associated
with Ankylosing Spondylitis
(Roldan et al. JACC
1998321397)

• TEE findings
• Aortic root
• Thickening 61
• Increased stiffness 61
• Dilatation 25
• Valve thickening
• aortic valve 41, mitral valve 34
• Nodularities of the aortic cusp and basal
  thickening of the anterior mitral valve leaflet
  (subaortic bump)
• Valve regurgitation 50 (moderate)

19
Aortic Root Disease and Valve Disease Associated
with Ankylosing Spondylitis

• No correlation with clinical features of AKS
• Evolution (Roldan et al. JACC 1998321397)
• 39-mon F/U of 25 patients
• New abnormalities 6 (24)
• Progression of valve regurgitation 3 (12)
• Resolved 5 (20)
• Therapy
• Questionable role of corticosteroid
• Aortic and mitral valve replacement in pts with
  severe aortitis and valvular dysfunction
• Prophylactic antithrombotic therapy
• Antibiotic prophylaxis for infective endocarditis
  in pts with moderate valvular regurgitation

20
Cardiac Disease Associated with Rheumatoid
Arthritis

• Pericarditis
• Autopsy studies 40
• Echo studies 50
• Clinically significant pericardial effusion lt 3
• Valvular heart disease
• Coronary arteritis
• Myocarditis
• Conduction disturbance

21
Valvular Heart Disease Associated with Rheumatoid
Arthritis

• Valvular diseases
• Valvular thickening
• Valvular regurgitation
• Valvular granulomas
• Prevalence
• Autopsy studies 23 to 75
• Echo studies
• TTE 30
• TEE Thickening 66
• Valvular nodules 50
• Valvular regurgitation 13

22
Valvular Nodules Associated with Rheumatoid
Arthritis

• Unique to RA
• Small (lt0.5 cm2)
• Oval in shape
• Well-defined border
• Homogenous reflectance
• Not calcified
• Usually single

(Cardiol Clin 199816531)
23
Valvular Heart Disease Associated with Rheumatoid
Arthritis

• Correlation with clinical features
• A higher prevalence of valvular disease in pts
  with erosive polyarticular and nodular disease,
  systemic vascularitis, and high titers of RA
  factor
• No association with the pts age, duration of RA,
  or peripheral nodular disease
• Therapy
• A few cases of significant improvement of severe
  valvulitis with the use of steroids or cytotoxic
  therapy
• Mitral and aortic valve replacement in severe
  regurgitation

24
Cardiac Disease Associated with Scleroderma

• Pericarditis
• Myocardial disease
• Myocardial fibrosis, myocarditis
• CHF 5
• Conduction disturbance
• Pulmonary hypertension
• One of the major causes of death
• Valvular heart disease
• Coronary artery disease

25
Valvular Heart Disease Associated with Scleroderma

• Limited echocardiographic data
• Nonspecific thickening of the mitral or aortic
  valve
• A high prevalence of MVP (67)
• Aortic valvulitis with a perforated cusp and
  severe regurgitation
• Noninfective mitral valve vegetations similar to
  those of SLE

(Cardiol Clin 199816531)
26
Cardiac Disease Associated with Polymyositis and
Dermatomyositis

• Myocarditis
• Pericarditis
• Mitral valve prolapse up to 50

27
Cardiac Disease Associated with Mixed Connective
Tissue Disease

• Pericarditis
• Pulmonary hypertension and
• cor pulmonale
• Mitral valve prolapse up to 32
• Verrucous thickening of the mitral valve and
  mitral regurgitation

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Summary

• Valvular abnormalities unique to a specific
  disease
• Libman-Sacks vegetation SLE
• Valve nodules RA
• Subaortic bump AKS
• Differential diagnosis
• Infective endocarditis
• Rheumatic valvular disease
• Degenerative valvular disease
• Echocardiography, especially TEE, has the
  potential to redefine the prevalence rates and to
  characterize better the cardiac abnormalities
  associated with connective tissue diseases.